This article describes a case of osteopathia striata with cranial sclerosis. The patient also has multiple sclerosis. The symptomatology includes a right sided conduction deafness and a left maxillar nerve deficit, which were both attributed to the bone disorder. The authors review the literature of this rare genetic syndrome and pay special attention to the neurological manifestations. These mainly consist of hearing loss, mental subnormality and occasionally the involvement of other cranial nerves. The bone scan in their patient shows hyperactivity in the left skull base region. This finding provides further evidence that, at least in some instances, the bone disorder has a progressive course.